Evaluation of treatment results in multifocal primary cutaneous anaplastic large cell lymphoma: report of the Dutch Cutaneous Lymphoma Group

BackgroundThere is no consensus on the treatment of multifocal primary cutaneous anaplastic large cell lymphoma (C-ALCL). Radiotherapy (RT) and methotrexate (MTX) are the current treatment options, but their efficacy is unknown. Recently, targeted therapies showed promising results in C-ALCL, and may therefore be an attractive first choice of treatment.ObjectivesTo assess the efficacy of conventional treatment strategies for patients with multifocal C-ALCL, and to define which patients may require novel targeted therapies.MethodsIn this multicentre study, treatment was evaluated in patients initially presenting (n = 24) or relapsing with multifocal C-ALCL (n = 17; 23 relapses). Distinction was made between patients with five or less lesions (n = 36) and more than five lesions (n = 11).ResultsTreatments most commonly used were RT (n = 21), systemic chemotherapy (n = 9) and low-dose MTX (n = 7) with complete response rates of 100%, 78% and 43%, respectively, and an overall response rate of 100%, 100% and 57%, respectively. Four patients showed complete spontaneous regression. In total, 16 of 24 patients (67%) first presenting with multifocal C-ALCL relapsed, including all five patients initially treated with CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone). Compared with patients presenting with two to five skin lesions, patients presenting with more than five lesions had a higher chance of developing extracutaneous relapse (56% vs. 20%) and more often died of lymphoma (44% vs. 7%).ConclusionsPatients with five or less lesions should be treated with low-dose RT (2 x 4 Gy). Maintenance low-dose MTX (20 mg weekly) is a suitable option in patients with more than five lesions. Targeted therapies may be considered in rare patients who are refractory to MTX or patients developing extracutaneous disease.What's already known about this topic?There is no consensus on the treatment of multifocal primary cutaneous anaplastic large cell lymphoma (C-ALCL). New targeted therapies are evaluated without knowledge of the efficacy of conventional therapies.What does this study add?This study evaluated the efficacy of conventional therapies for multifocal C-ALCL. Radiotherapy and low-dose methotrexate are suitable options in patients with five or less and more than five lesions, respectively. Targeted therapies may be considered in rare patients who are refractory to methotrexate or developing extracutaneous disease.Linked Comment:Kempf. Br J Dermatol 2018; 179:565-566. Respond to this article